AB0740 INTERSTITIAL LUNG DISEASE SYSTEMIC SCLEROSIS: AN EAST-EUROPEAN EUSTAR CENTER EXPERIENCE
Authors: D. M. Artene, C. Ancuta
Journal: Annals of the Rheumatic Diseases (2022)

Background Systemic sclerosis (SSc) is a complex autoimmune connective tissue disorder defined by multifaceted visceral involvement. Still considered the leading cause of morbidity and mortality, interstitial lung disease (ILD) remains one the most common complication of SSc that involves vascular, immunological, and fibrotic processes. Objectives We aimed to assess the relationship between demographic, clinical features and risk factors for the development of lung fibrosis in patients with SSc as well as the specific management. Methods We performed a retrospective observational study in a series of 45 patients with SSc and ILD followed-up in East-European EUSTAR center (Department of Rheumatology 2, Clinical Rehabilitation Hospital, Iasi, Romania). We analyzed the frequency of known risk factors associated with progressive interstitial lung disease such as diffuse cutaneous SSc, male sex, presence of anti-topoisomerase I and absence of anti-centromere antibodies as well as risk factors for mortality in SSc-ILD including older age, male sex, extent of disease on high-resolution computed tomography (HRCT), lower pulmonary function tests (PFTs), lower diffusing capacity of the lung for carbon monoxide (DLCO). Results The study included 35 females, 10 males, with the mean age of 55.5 years. The diagnosis of interstitial lung disease was based on HRCT findings (bibasilar ground glass opacities, crazy-paving pattern, nodules, honeycombing and traction bronchiectasis, mosaic pattern, air trapping, pulmonary artery and lymph node enlargement). Symptoms of the ILD (dry cough, different stages of dyspnea, VELCRO crackles) were reported in 14 cases (25.45%). Furthermore, 25 patients had a significant decline in PFTs and DLCO, being associated with extensive pulmonary fibrosis on HRCT (p&lt;0.05%). Almost all patients with SSc-ILD had diffuse cutaneous involvement and anti-Scl-70 antibodies positivity. Despite identifying a higher percentage of women with ILD (35/45, 63.63%), the extent and progression of pulmonary fibrosis was more significant in males. The treatment for SSc-ILD with ongoing evidence of disease progression based on PFT decline or radiographic deterioration has focused on immunosuppressive therapies, particularly cyclophosphamide and only in selected cases on antifibrotic agents. Conclusion It is important to identify the risk factors for developing ILD, in order to complete the diagnostic and staging parameters early so that the treatment can be initiated as soon as possible in progressive pattern. Our data showed that the extent of pulmonary fibrosis in HRCT was associated with low values of PFTs and DLCO, and men had a more severe prognostic than women. ILD-SSc was more frequent in patients with diffuse cutaneous involvement than limited cutaneous subtype, and in those with anti-Scl-70 positivity. References [1]Perelas A, Silver RM, Arrossi A et al, Systemic-sclerosis associated interstitial lung disease, Lancet Respir Med 2020, https://doi.org/10.1016/S2213-2600 (19)30480-1 [2]N Tanaka, Y Kunihiro M Kubo et al, HRCT findings of collagen vascular disease-related interstitial pneumonia (CVD-IP): a comparative study among individual underlying diseases, Clinical Radiology 73 (2018) 833.e1-833.e10, https://doi.org/10.1016/j.crad.2018.04.017 [3]Dinesh Khanna, Donald P. Tashkin, Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease, Am J Respir Crit Care Med. 2020 Mar 15; 201(6): 650–660. DOI: https://dx.doi.org/10.1164%2Frccm.201903-0563CI [4]Vincent Cottin, Kevin K. Brown, Interstitial lung disease associated with systemic sclerosis (SSc-ILD), Cottin and Brown Respiratory Research (2019) 20:13 https://doi.org/10.1186/s12931-019-0980-7 [5]Padmini Khedoe, Emiel Marges, Pieter Hiemstra, Interstitial Lung Disease in Patients With Systemic Sclerosis: Toward Personalized-Medicine-Based Prediction and Drug Screening Models of Systemic Sclerosis-Related Interstitial Lung Disease (SSc-ILD). Front Immunol. 2020 Sep 4;11:1990, doi: 10.3389/fimmu.2020.01990. Disclosure of Interests None declared